These results suggest that EUS-FNA is of value as a new, less-invasive approach for tissue diagnosis of hilar strictures of unknown cause. It was technically feasible without significant risks, when other diagnostic tests were inconclusive and was able to change preplanned management in about half of the patients.
Resectional surgery offers a curative intent and a survival benefit for patients with hilar cholangiocarcinoma, but is associated with high morbidity. Since morphological imaging cannot solve differential diagnosis preoperatively, in order to exclude patients inappropriate to this aggressive surgery, we evaluated the impact of functional imaging using fluorodeoxyglucose positron emission tomography (FDG PET) in the detection of cholangiocarcinoma and its usefulness in the differentiation from benign Klatskin tumour-mimicking lesions. Fifteen consecutive patients aged 47-78 years underwent standardized whole-body FDG PET with attenuation correction before potentially curative surgery using a conventional full-ring PET scanner with an axial field-of-view of 16.2 cm. FDG PET was evaluated visually and semiquantitatively using tumour-to-background ratios (T/B) ratios. All lesions were evaluated histopathologically. FDG PET presumed to be indicative for carcinoma was positive in 12 of 15 patients, true positive in 10 (T/B ratio, 3.2+/-1.9) and false positive in two of them (T/B ratios, 2.1 and 2.8) with Klatskin tumour-mimicking lesions. While all true positive PET results were seen in the tubular type of cholangiocarcinoma with a high amount of tumour cells and only low production of mucus, a false negative FDG PET in three patients was observed in mucinous adenocarcinoma. Additionally, FDG PET detected locoregional lymph nodes in two patients and distant metastases in a further three patients. Due to false positive results FDG PET does not allow the differentiation of benign from malignant lesions, and FDG PET should be avoided in patients with mucinous cholangiocarcinoma. However, FDG PET may have significant influence on the treatment strategy in as much as 20% of the patients, since it may detect distant metastases.
In primary hyperoxaluria type 1 (PH 1), deficiency or mis‐targeting of hepatic alanine glyoxylate aminotransferase (AGT) results in over‐production of oxalate and hyperoxaluria, leading to nephrocalcinosis and development of end‐stage renal disease (ESRD) in the majority of patients. Renal transplantation (Tx) alone carries a high risk of disease recurrence as the metabolic defect is not cured. Therefore, combined liver/kidney Tx is recommended for patients with ESRD. An alternative approach is to cure PH 1 by pre‐emptive isolated liver Tx (PLTx) before ESRD has occurred, but this approach has been carried out only occasionally and there are no uniformly accepted recommendations concerning the timing of this procedure. We report follow‐up 3–5.7 yr after performing successful PLTx in four children (at the age of 3–9 yrs) with PH 1 prior to the occurrence of ESRD (glomerular filtration rate [GFR] range 27–98 mL/min/1.73 m2). There was no mortality or long‐term morbidity associated with the Tx procedure. Plasma and urinary oxalate levels normalized rapidly within 4 weeks, and renal function did not deteriorate under immunosuppression, even in one patient with advanced chronic renal failure (GFR 27 mL/min/1.73 m2) who showed a stable course for more than 5.7 yrs. Although treatment must be individualized in this severe metabolic disorder, and PLTx has to be regarded as an invasive procedure, we consider that PLTx should be offered and considered early in the course of PH 1. PLTx cures the metabolic defect in PH 1 and can help to prevent, or at least delay, the progression to ESRD and systemic oxalosis.
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